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Congenital hyperinsulinism treated by surgical resection of the hyperplastic lesion which had been preoperatively diagnosed by 18F-DOPA PET examination in Japan: a nationwide survey

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Abstract

Purpose

Congenital hyperinsulinism is a rare disease, and the newly developed 18 fluoro-L-dihydroxyphenylalanine-positron emission tomography (18F-DOPA PET) examination can detect hyperplastic lesions. Our purpose was to report the results of a nationwide survey on surgical treatment of congenital hyperinsulinism in Japan.

Methods

A questionnaire was sent to the 159 accredited and affiliated training institutes certified as pediatric surgical institutes by the Japanese Association of Pediatric Surgeons, asking if they had encountered patients who underwent surgical treatment for congenital hyperinsulinism after 18F-DOPA PET examination from 2000 to 2017. Six institutes answered that they had treated such cases, and the total number of cases was 14.

Results

18F-DOPA PET examination detected the focal lesion in 12 of the 14 cases. 18F-DOPA PET examination could accurately determine the site of the hyperplastic lesion in the pancreas in 11 (91.7%) of the 12 cases. All cases underwent surgical resection of the hyperplastic lesion at under 2 years of age.

Conclusion

Surgical resection of a focal hyperplastic lesion in the pancreas was a safe and effective treatment if the hyperplastic lesion was a focal lesion. However, it is necessary to check the exact distribution of the lesion by intraoperative pathologic examination of frozen sections.

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Acknowledgements

This research was accomplished with the support of a grant from The Ministry of Health, Labor and Welfare of Japan (H28-Nanziseitou(nan)-Ippan-021). The authors wish to thank the doctors at the Japanese accredited institutions and affiliated training institutions who replied to the questionnaires, and especially the doctors at the following six institutes for answering the detailed second questionnaire: Department of Pediatric Surgery, Kyoto University Hospital; Department of Pediatric Surgery, Developmental Surgery, and Intestinal Transplant Surgery, Kyusyu University Hospital; Department of Pediatric Surgery, Shizuoka Children’s Hospital; Department of Surgery, Kanagawa Children’s Medical Center; Department of Pediatric Surgery, Japan Community Health Care Organization Kyusyu Hospital; Division of Surgery, National Center for Child Health and Development.

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Authors and Affiliations

  1. Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, 2-10-1 Okura Setagaya-ku, Tokyo, 157-8535, Japan

    Yutaka Kanamori & Toshihiko Watanabe

  2. Division of Pediatric Endocrinology and Metabolism, Children’s Medical Center, Osaka City General Hospital, Osaka, Japan

    Tohru Yorifuji

  3. Department of Pediatrics, Kizawa Memorial Hospital, Gifu, Japan

    Michiya Masue

  4. Department of Pediatric Surgery, Tohoku University, Miyagi, Japan

    Hideyuki Sasaki & Masaki Nio

Authors
  1. Yutaka Kanamori
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  2. Toshihiko Watanabe
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  3. Tohru Yorifuji
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  4. Michiya Masue
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  5. Hideyuki Sasaki
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  6. Masaki Nio
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Correspondence to Yutaka Kanamori.

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The authors declare that they have no conflict of interest.

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Kanamori, Y., Watanabe, T., Yorifuji, T. et al. Congenital hyperinsulinism treated by surgical resection of the hyperplastic lesion which had been preoperatively diagnosed by 18F-DOPA PET examination in Japan: a nationwide survey. Pediatr Surg Int 34, 1093–1098 (2018). https://doi.org/10.1007/s00383-018-4315-4

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  • DOI: https://doi.org/10.1007/s00383-018-4315-4

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